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 Table of Contents  
LETTER TO EDITOR
Year : 2019  |  Volume : 6  |  Issue : 1  |  Page : 53-54

Extragenital lichen sclerosus et atrophicus mimicking mycosis fungoides histologically


Department of Dermatology, HBT Medical College and Dr. RN Cooper Hospital, Mumbai, Maharashtra, India

Date of Web Publication13-Jun-2019

Correspondence Address:
Kinjal Deepak Rambhia
Department of Dermatology, HBT Medical College and Dr. RN Cooper Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_52_18

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How to cite this article:
Rambhia KD, Agrawal A, Makhecha MB. Extragenital lichen sclerosus et atrophicus mimicking mycosis fungoides histologically. Indian J Dermatopathol Diagn Dermatol 2019;6:53-4

How to cite this URL:
Rambhia KD, Agrawal A, Makhecha MB. Extragenital lichen sclerosus et atrophicus mimicking mycosis fungoides histologically. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2019 Sep 22];6:53-4. Available from: http://www.ijdpdd.com/text.asp?2019/6/1/53/260190



Sir,

A 35-year-old male presented with multiple asymptomatic light-colored lesions over the trunk and bilateral lower extremity for 6 months. There was no history of appearance of red-raised skin lesions before the appearance of the white-colored lesions. There was no history of application of irritants before the appearance of the lesions. Cutaneous examination revealed multiple white atrophic macules, few coalescing to form patches over the bilateral lower extremities and few scattered lesions over the trunk [Figure 1]. Histopathological examination revealed lichenoid infiltrate of the lymphocytes in the papillary dermis. There were multiple lymphocytes located in the lower layers of the epidermis and at the dermoepidermal junction [Figure 2] and [Figure 3]. These lymphocytes showed the nuclei surrounded by a clear halo. The stratum corneum was hyperplastic with a relative reduced stratum spinosum focally [Figure 4]. Homogenization of collagen bundles and thickened collagen bundles were only seen in one focus. The papillary dermis showed few melanophages. After clinicopathologcial correlation, the patient was diagnosed with a case of extragenital lichen sclerosus et atrophicus (LSA). This case illustrates that mild lichenoid dermatitis, tagging of lymphocytes at the dermoepidermal infiltrate, basilar epidermotropism, and haloed lymphocytes at the dermoepidermal junction may be seen in early lesions of LSA, highlighting the importance of clinical correlation in such cases.
Figure 1: White atrophic macules and patches over the bilateral lower extremities

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Figure 2: Collections of lymphocytes at the dermoepidermal junction

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Figure 3: Tagging of lymphocytes at the basal layer with coarse papillary dermal collagen

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Figure 4: Thickened stratum corneum and thinning of the stratum spinosum with effaced rete ridges, vacuolar change at the dermoepidermal junction. Note the slight thickening of the collagen bundles of the papillary dermis and the formation of trilayered appearance

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LSA is a chronic sclerosing inflammatory dermatosis of unknown etiology commonly affecting the anogenital skin. Extragenital LSA though encountered less frequently is characterized clinically by shiny, white, atrophic macules and papules coalescing to form plaques. The most common sites of extragenital LSA are the trunk and thighs. The salient histological findings of LSA are hyperkeratosis with follicular plugging, atrophy of the stratum malphigii, edema, and homogenization of the collagen in the upper dermis. Classical lesions show a trilayered or striped appearance comprising compact orthokeratosis and atrophied epidermis, a pale superficial papillary dermis, and inflammatory infiltrate in the mid dermis.[1]

Rarely, LSA may show histological findings of tagging of lymphocytes in the basal layer, mild vacuolar interface dermatitis, and lymphocytic exocytosis.[2] Early mycosis fungoides (MF) is defined by the presence of single lymphocyte along the basal layer of the epidermis, atypical haloed lymphoid cells, mild vacuolar interface dermatitis, and by the presence of papillary dermal fibrosis.[3]

Histopathologically, in cases of LSA with tagging of lymphocytes in the basal layer of the epidermis and exocytosis, in the absence of classical features of LSA, it is very difficult to rule out MF.

Genital LSA is known to mimic MF histologically.[4] Extragenital LSA mimicking MF has previously been reported by Suchak et al.[5]

Despite the histologic resemblance of MF and LSA, several features help to differentiate between these conditions. Haloed lymphocytes are less common in LSA, and epidermotropic lymphocytes are confined to the lower layers of the epidermis (in contrast to the pagetoid spread of lymphocytes, involving the upper layers as well in MF). Occasional dyskeratotic keratinocytes, vacuolar, interface dermatitis may be found more commonly in LSA [Table 1].
Table 1: Histopathological differences of atypical lichen sclerosus et atrophicus and mycosis fungoides

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Currently, the true frequency of this atypical presentation of LSA encountered by dermatopathologists is not known; though it is not very rare. Awareness of this atypical presentation of early LSA is necessary to avoid an erroneous diagnosis of MF. Clinicopathological correlation is required in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Winfield H, Jaworsky C. Connective tissue disease. In: Elder DE, editor. Histopathology of the Skin. Philadelphia: Wolters and Kluvers; 2015. p. 355-9.  Back to cited text no. 1
    
2.
Yegappan S, Lee MW, Amin MB, Lymphocytic epidermotropism (LET) simulating mycosis fungoides (MF) in foreskin, vulva and extragenital lichen sclerosus et atrophicus. Mod Pathol 1997;10:48.  Back to cited text no. 2
    
3.
Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al. Defining early mycosis fungoides. J Am Acad Dermatol 2005;53:1053-63.  Back to cited text no. 3
    
4.
Citarella L, Massone C, Kerl H, Cerroni L. Lichen sclerosus with histopathologic features simulating early mycosis fungoides. Am J Dermatopathol 2003;25:463-5.  Back to cited text no. 4
    
5.
Suchak R, Verdolini R, Robson A, Stefanato CM. Extragenital lichen sclerosus et atrophicus mimicking cutaneous T-cell lymphoma: Report of a case. J Cutan Pathol 2010;37:982-6.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

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